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  • Title: Lysosomal storage of subunit c of mitochondrial ATP synthase in Batten's disease (ceroid-lipofuscinosis).
    Author: Hall NA, Lake BD, Dewji NN, Patrick AD.
    Journal: Biochem J; 1991 Apr 01; 275 ( Pt 1)(Pt 1):269-72. PubMed ID: 1826833.
    Abstract:
    Immunochemical studies demonstrate that subunit c of mitochondrial ATP synthase is stored in the late-infantile, juvenile and adult forms of Batten's disease. It does not accumulate in the infantile form, or in other conditions involving lysosomal hypertrophy. These results suggest that the defective metabolism of subunit c is central to the pathogenesis of these three forms of Batten's disease.
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